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March 14, 2005 Monday 7:44 AM EST
LENGTH: 179 words
HEADLINE: Scientists silence, replace an ALS gene
DATELINE: LAUSANNE, Switzerland, March 14

Swiss scientists have delayed the onset and progression of inherited Lou Gehrig's disease in mice by silencing a mutant gene that triggers it.

Amytrophic lateral sclerosis or ALS is a fatal disorder that attacks the motor neurons controlling muscles.

Some 5,000 Americans are diagnosed with ALS every year, with most cases having no known cause. Roughly 5 percent to 10 percent of ALS cases are inherited, however, and genetic links have been identified for about 20 percent of those cases.

In this study, researchers not only were able to silence one of the disease-causing genes, they also delivered a normal version of the gene to motor neuron cells.

"This is the first proof of principle in the human form of a disease of the nervous system in which you can silence the gene and at the same time produce another normal form of the protein," wrote Patrick Aebischer, president of the Federal Institute of Technology in Lausanne, where the research was done.

The study will be detailed in the April issue of Nature Medicine, and is in the journal's advanced online publication.